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先天性巨结肠症25年的治疗经验。

Twenty-five years' experience with Hirschsprung's disease.

作者信息

Foster P, Cowan G, Wrenn E L

机构信息

Department of General Surgery, Rush-Presbyterian-St Luke's Medical Center, Chicago, IL 60612.

出版信息

J Pediatr Surg. 1990 May;25(5):531-4. doi: 10.1016/0022-3468(90)90566-r.

DOI:10.1016/0022-3468(90)90566-r
PMID:2352087
Abstract

Sixty-three patients with biopsy-proven Hirschsprung's disease were diagnosed at LeBonheur Children's Medical Center, Memphis, TN between 1955 and 1980. Fifty-eight of these patients had pull-through procedures performed by three similarly trained pediatric surgeons. The follow-up was 100 percent, averaging 8 years from initial diagnosis. Demographics, surgical procedures performed, and complications are reviewed. Significant findings are (1) anastomotic strictures occurred most frequently when the level of aganglionosis was at the sigmoid colon; (2) postoperative encopresis was most likely when the endorectal pull-through procedures were performed before the age of 10 months; and (3) with selective use of colostomies or enterostomies performed prior to the pull-through procedure, the incidence of enterocolitis was low, with 0% mortality. On the basis of these findings, we recommend that, when the most proximal level of aganglionosis is the sigmoid colon, it is important to critically inspect the angulation of mesenteric blood vessels and viability of the splenic flexure pull-through colon segment to prevent ischemia and therefore anastomotic strictures. The endorectal pull-through procedure should be delayed until after 10 months of age. Infants with Hirschsprung's disease should have a colostomy or enterostomy prior to a pull-through procedure. Patients diagnosed at 10 months of age or more, who have not had earlier bouts of enterocolitis, are not low percentile weight, and are without signs of severe obstruction, are candidates for pull-through without a prior fecal diversion procedure.

摘要

1955年至1980年间,田纳西州孟菲斯市勒博内尔儿童医院诊断出63例经活检证实的先天性巨结肠症患者。其中58例患者接受了由三位训练相似的小儿外科医生实施的拖出术。随访率为100%,从初次诊断起平均随访8年。本文回顾了患者的人口统计学资料、所实施的外科手术及并发症情况。重要发现如下:(1)无神经节细胞症位于乙状结肠时,吻合口狭窄最为常见;(2)直肠内拖出术在10个月龄之前实施时,术后大便失禁最为常见;(3)在拖出术之前选择性地施行结肠造口术或肠造口术,小肠结肠炎的发生率较低,死亡率为0%。基于这些发现,我们建议,当无神经节细胞症最近端位于乙状结肠时,至关重要的是要严格检查肠系膜血管的角度以及脾曲拖出结肠段的活力,以防止缺血进而预防吻合口狭窄。直肠内拖出术应推迟至10个月龄之后进行。先天性巨结肠症婴儿在拖出术之前应进行结肠造口术或肠造口术。10个月龄及以上诊断出的患者,若未曾有过小肠结肠炎发作史、体重百分位数不低且无严重梗阻体征,则可在不进行先前粪便转流手术的情况下进行拖出术。

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